Pediatric Orthopaedic Venous Thromboembolism: A Systematic Review Investigating Incidence, Risk Factors, and Outcome.

Background: There is growing evidence of increased venous thromboembolism (VTE) incidence in children with trauma or infection. The purpose of this study was to conduct a systematic review of existing literature related to VTE in the pediatric orthopaedic population, to estimate the overall incidence of VTE and identify risk factors associated with this condition. Methods: A systematic review of the available literature was performed to identify articles that described VTE in pediatric orthopaedic surgery or admission. Literature queries were performed to identify articles published from 1980 to 2021 that included patients ≤21 years of age. A stepwise search strategy of 5 electronic databases yielded 1,426 articles, which were filtered by 2 reviewers to identify 30 articles for full-text review. The primary aim was to determine the rate of VTE, and the secondary aim was to identify risk factors for VTE. The pooled incidence of VTE was estimated and reported in cases per 10,000. Studies were stratified by study size, by trauma versus elective surgery, and by orthopaedic subspecialty. Results: The 30 articles reported 3,113 VTE events in 2,467,764 pediatric patients (including those with non-orthopaedic conditions), for a pooled VTE incidence of 20 events (95% confidence interval [CI] = 10.8 to 37.2) per 10,000. Four of the studies were excluded for incomplete data or high heterogeneity. The remaining 26 studies had 850,268 orthopaedic patients with 1,108 cases of VTE, for a pooled VTE incidence of 16.6 events (95% CI = 9.1 to 30.5) per 10,000. Studies with <10,000 patients and those involving a diagnosis of trauma had the highest VTE incidence when stratification was performed. The most frequently analyzed risk factors in 15 available studies included age, sex, obesity/body mass index, type of surgery, and use of a central venous catheter. Conclusions: This systematic review indicated that the risk of VTE associated with pediatric orthopaedic surgery or admission remains low, at <17 events per 10,000 cases. However, orthopaedic surgeons should be aware of the most common risk factors associated with pediatric orthopaedic VTE and should pay special attention to traumatic etiologies, as these yielded the highest incidence. Levels of Evidence: Prognostic Level III. See Instructions for Authors for a complete description of levels of evidence.

Reliability of the Masada Classification for Forearm Involvement in Patients With Hereditary Multiple Osteochondromas (HMO).

BACKGROUND: Classifications describing forearm lesions in patients with Hereditary Multiple Osteochondromatosis (HMO) have been used to recommend surgical intervention and stratify outcomes; however, there is no consensus on which classification offers greater reliability. The purpose of this study was to determine the reliability of the Masada classification and newer classifications among pediatric hand surgeons. METHODS: One hundred one patients with HMO between June 2014 and October 2019 were enrolled in the Congenital Upper Limb Differences (CoULD) Registry. Of those, 67 patients with 101 forearms were included. Four pediatric hand surgeons from the CoULD study group undertook an online evaluation. Each rater classified radiographs according to the Masada classification. Six weeks later, raters were asked to reclassify images according to the Masada, Gottschalk, and Jo classifications. Rater agreement for these classifications was assessed by estimating Fleiss kappa along with a 95% CI. RESULTS: Interrater agreement for Masada classification after the first reading was poor (κ=0.35; 95% CI=0.30-0.41) across all raters. Interrater agreement across the 4 raters decreased for the Masada classification from the first to the second reading (κ=0.35 vs 0.21; P <0.001). Intrarater agreement for the Masada classification ranged from 0.32 to 0.63 from the first to the second study reading. Gottschalk and Jo classifications yielded significantly better interrater agreement compared with Masada (κ=0.43 vs 0.21; P <0.001). Unclassifiable cases were highest in the Masada classification (34% to 44%) and lower in the Jo (17%) and Gottschalk (14%) classifications. CONCLUSION: Despite wide use, the Masada classification was found to have low reliability when classifying forearm deformities in HMO. Gottschalk offered more options for location, yet lacked deformity description including radial head dislocation. Jo classification offered more locations than Masada and incorporated radial head dislocation in some patterns. Based on the shortcomings in all 3 classification systems, the development of a more inclusive and reliable classification is warranted. LEVEL OF EVIDENCE: Level II; Diagnostic.

How Risky Are Risk Factors? An Analysis of Prenatal Risk Factors in Patients Participating in the Congenital Upper Limb Differences Registry.

Purpose: Risk factors for congenital upper limb differences (CoULDs) are often studied at the general population level. The CoULD registry provides a unique opportunity to study prenatal risk factors within a large patient sample. Methods: All patients enrolled between June 2014 and March 2020 in the prospective CoULD registry, a national multicenter database of patients diagnosed with a CoULD, were included in the analysis. We analyzed self-reported, prenatal risk factors, including maternal smoking, alcohol use, recreational drug use, prescription drug use, gestational diabetes mellitus (GDM), and gestational hypertension. The outcome measures included comorbid medical conditions, proximal involvement of limb difference, bilateral involvement, and additional orthopedic conditions. Multivariable logistic regression was used to analyze the effect of the risk factors, controlling for sex and the presence of a named syndrome. Results: In total, 2,410 patients were analyzed, of whom 72% (1,734) did not have a self-reported risk factor. Among the 29% (676) who did have at least 1 risk factor, prenatal maternal prescription drug use was the most frequent (376/2,410; 16%). Maternal prescription drug use was associated with increased odds of patient medical comorbidities (odds ratio [OR] = 1.43, P = .02). Gestational diabetes mellitus was associated with increased odds of comorbid medical conditions (OR = 1.58, P = .04), additional orthopedic conditions (OR = 1.51, P = .04), and proximal involvement (OR = 1.52, P = .04). Overall, reporting 1 or more risk factors increased the odds of patient comorbid medical conditions (OR = 1.42, P < .001) and additional orthopedic conditions (OR = 1.25, P = .03). Conclusions: Most caregivers (72%) did not report a risk factor during enrollment. However, reporting a risk factor was associated with patient medical and orthopedic comorbidities. Of note, GDM alone significantly increased the odds of both these outcome measures along with proximal limb differences. These findings highlight the ill-defined etiology of CoULDs but suggest that prenatal risk factors, especially GDM, are associated with a higher degree of morbidity. Type of study/level of evidence: Prognostic III.

Congenital Upper Limb Differences Registry (CoULD): Registry Inclusion Effect.

PURPOSE: To advance the understanding of the epidemiology and treatment outcomes of congenital upper limb differences, a multicenter registry for Congenital Upper Limb Differences (CoULD) was established. After 4 years of recruitment, we sought to examine whether the relative frequency of congenital conditions compares with prior cross-sectional research and how the data have matured over time by (1) comparing our registry population with previous studies in similar populations and (2) evaluating the change over time of relative frequencies of selected conditions within the CoULD registry cohort, specifically to investigate for registry inclusion effects. METHODS: Data from the 2 founding centers in the CoULD registry were analyzed over a 4-year period. We compared patients included in the CoULD registry against 2 prior studies by matching each condition according to the Oberg-Manske-Tonkin classification system. The relative frequency of 4 representative conditions was calculated to evaluate change over time and to determine when the inception cohort effect diminished. RESULTS: The CoULD cohort of 1,381 patients was found to have notable differences compared with a 1-year cross-sectional cohort from the U.S. Midwest and a Swedish birth registry. Each of these registries had differences from the CoULD population in prevalence for approximately 33% of the diagnosis categories. The CoULD registry identified and included more pathologies of late presentation and those that do not commonly require surgical care. Changes in relative frequencies of incident and prevalent conditions, the registry inclusion effect, occurred early and stabilized by the third year. CONCLUSIONS: The CoULD registry captures a different relative frequency of conditions than prior studies in similar populations. The findings highlight the CoULD registry may be a more accurate representation of clinical practice in tertiary referral centers; however, it is important to note that there was a registry inclusion effect identified. CLINICAL RELEVANCE: Inclusion criteria are an important consideration with any longitudinal data collection method and data should display stability prior to registry reporting.

Association of Radial Longitudinal Deficiency and Thumb Hypoplasia: An Update Using the CoULD Registry.

BACKGROUND: Deficiency of the radial aspect of the forearm and hand is the most common congenital longitudinal deficiency of the upper limb. Radial longitudinal deficiency is associated with several named syndromes. The purpose of the present study was to explore patterns of radial longitudinal deficiency and thumb hypoplasia in syndromes and to examine the severity of these differences across various syndromes. METHODS: Data were collected from the Congenital Upper Limb Differences (CoULD) registry. Congenital differences are classified in the registry with use of the Oberg-Manske-Tonkin (OMT) classification system. Diagnosis of a syndrome by a physician as noted in the CoULD registry was recorded. Thumb deficiency and radial deficiency were classified according to the modified versions of the Blauth criteria and the Bayne and Klug criteria, respectively. RESULTS: We identified 259 patients with 383 affected limbs with radial deficiency. Eighty-three of these patients had a diagnosed syndrome. The severity of radial deficiency was correlated with the severity of thumb deficiency. The Kendall tau coefficient indicated significant correlation between radial severity and thumb severity (tau = 0.49 [95% confidence interval = 0.40 to 0.57]; p < 0.05). Subjects with a syndrome were twice as likely to have bilateral deficiency and 2.5 times more likely to have both radial and thumb deficiency compared with subjects without a syndrome. Subjects with VACTERL syndrome (vertebral defects, anal atresia, cardiac anomalies, tracheoesophageal fistula, renal anomalies, and limb defects) had patterns of thumb and radial deficiency similar to the general cohort, whereas subjects with Holt-Oram syndrome, TAR (thrombocytopenia absent radius) syndrome, and Fanconi anemia demonstrated varied presentations of thumb and radial deficiency. CONCLUSIONS: The present study investigated the characteristics of patients with radial longitudinal deficiency and thumb hypoplasia. Our results support the findings of previous research correlating the severity of radial deficiency with the severity of thumb deficiency. Furthermore, we identified characteristic features of patients with radial longitudinal deficiency and associated syndromes.

Pollicization of the Index Finger in the United States: Early Readmission and Complications.

PURPOSE: Pollicization is a well-accepted treatment for thumb hypoplasia, yet little is known about the reasons why patients return early in the postoperative period and complications that occur after surgery. The purpose of this investigation was to describe 30-day returns, readmission, and complication rates after pollicization in the United States. METHODS: A total of 459 pollicization procedures performed in 408 patients at 38 U.S. pediatric hospitals from 2003 to 2014 were identified using the Pediatric Health Information System database. A stepwise search strategy identified returns and readmissions within 30 days after pollicization to quantify complications and/or additional procedures. Risk factors for readmission and complications were compared across groups using uni- and multivariable general linear modeling. RESULTS: There were 61 patients who returned to the hospital (emergency department, ambulatory surgery, or inpatient hospitalization) within 30 days of their pollicization. Of those, 39 patients returned for suture removal, cast changes, or other expected aspects of postoperative care. The remaining 22 patients had a total of 26 complications, accounting for a 4.8% complication rate. The majority had vascular complications and wound problems. Single complications ranging from wound infection to hemorrhage were found in 20 cases. Overall, 35 of the 61 returns were readmitted to the hospital for treatment of complications or additional procedures. There was no effect of age group, diagnosis, geographical region, or physician subspecialty on the likelihood of complication or readmission. CONCLUSIONS: Sixty-one patients returned within 30 days of their pollicization, and 22 presented with a complication (4.8%), most commonly vascular in nature. These baseline data are informative because they identify opportunities for future preventative measures and quality improvement. TYPE OF STUDY/LEVEL OF EVIDENCE: Therapeutic IV.

Intraobserver and Interobserver Reliability of the Oberg-Manske-Tonkin (OMT) Classification: Establishing a Registry on Congenital Upper Limb Differences.

BACKGROUND: The purpose of this investigation was to determine the reliability of the Oberg-Manske-Tonkin (OMT) classification system applied to patients enrolled in a prospective, multicenter cohort study of congenital upper limb differences. Our hypothesis was that the OMT classification would exhibit high intraobserver and interobserver reliability and thus would be an appropriate tool for the new Congenital Upper Limb Differences registry. METHODS: Four pediatric hand surgeons independently reviewed the medical records, clinical photographs, and radiographs of 60 randomly selected patients previously enrolled in the Congenital Upper Limb Differences registry. Patient's information was summarized and shared electronically (PowerPoint). Each rater classified each congenital anomaly according to the OMT classification system. Responses were recorded using a web-based data capture tool (REDCap). Three weeks later, the surgeons repeated the process. Intraobserver reliability for each rater was assessed using Fleiss' κ. Interobserver reliability was assessed using percent exact agreement (when all 4 raters were in agreement) as well as Fleiss' κ. RESULTS: Using the OMT classification, intraobserver reliability for the 4 pediatric hand surgeons showed almost perfect agreement, with κ values in the range of 0.89 to 0.93. Interobserver reliability demonstrated substantial agreement, with κ value of 0.79 (95% confidence interval, 0.77-0.82) in the first reading and 0.80 (95% confidence interval, 0.77-0.83) in the second reading. The highest possible agreement (κ=1) was seen for the following diagnoses: congenital dislocation of radial head (OMT I.A.2.v), Madelung deformity (I.A.2.vii), radial polydactyly (I.B.2.iii), triphalangeal thumb (I.B.2.iv), Kirner deformity (I.B.4.vi), and osteochondromatosis (3.B.4.i). CONCLUSIONS: The OMT classification of congenital upper limb differences exhibits substantial to almost perfect intraobserver and interobserver reliability among pediatric hand surgeons at different institutions. The OMT is therefore well-suited for classification of congenital upper limb anomalies in patients enrolled in a multicenter registry. LEVEL OF EVIDENCE: Level II-diagnostic.

Functional Impact of Congenital Hand Differences: Early Results From the Congenital Upper Limb Differences (CoULD) Registry.

PURPOSE: To characterize the functional, emotional, and social impact of congenital upper limb differences on affected children and families before treatment, using validated functional outcome instruments. METHODS: From June 2014 to March 2016, 586 children with congenital upper limb differences from 2 pediatric hospitals were enrolled in the Congenital Upper Limb Differences registry. Demographic, clinical, and radiographic data were collected, and diagnoses categorized according to the Oberg-Manske-Tonkin classification. Functional outcomes were assessed in 301 patients using the Pediatric Outcomes Data Collection Instrument (PODCI) and Patient-Reported Outcomes Measurement Information System (PROMIS) upper extremity (UE) function, pain, anxiety, depression, and peer relationships modules. RESULTS: The cohort had high median PODCI scores in all domains, ranging from 83 to 100 in children and adolescents. Patients had decreased PROMIS UE scores compared with population norms; however, they showed low scores for pain, anxiety, depression and higher scores in the peer relationship domain, respectively. Patients with entire limb involvement had higher PROMIS pain scores and lower PODCI UE and global functioning than those with differences limited only to the hand. Compared with those with bilateral involvement, patients with unilateral differences reported higher scores for PODCI sports global functioning, better PROMIS UE function, and lower pain scores. Additional orthopedic conditions and medical comorbidities negatively influenced all PODCI scores and PROMIS pain and UE function domains. CONCLUSIONS: Children with congenital hand differences report decreased upper limb function but better peer relationships and positive emotional states compared with population norms. CLINICAL RELEVANCE: The Congenital Upper Limb Differences registry is a valid source of information related to congenital upper limb differences in clinical practice. With continuous enrollment and longitudinal follow-up, the registry will increase the understanding of UE function and psychosocial aspects of health in pediatric population.

Assessment of Health Needs in Children with Congenital Upper Limb Differences in Nicaragua: Community Case Study.

Congenital anomalies are prevalent in Nicaragua, and disability is estimated to be 10% in the general population. We studied children with congenital upper limb differences, as they are vulnerable to disability. This case study documents a collaborative effort between American and Nicaraguan orthopedic surgeons to determine unmet health needs of children with congenital upper limb differences at Hospital Manuel de Jesus Rivera ("La Mascota" Hospital) in Nicaragua, with the goal of developing programs that successfully address these needs within the context of the priorities of the community. Participants were recruited during one of the biannual pediatric hand specialty clinics held by a partnership of pediatric hand surgeons and occupational therapists under the auspices of Health Volunteers Overseas (La Brigada de las Manos, or "La Brigada") and Nicaraguan orthopedic surgeons. Structured interviews were performed with 34 parents or caregivers of patients with the diagnosis of a congenital upper limb difference. Parents were asked to rank the social, economic, environmental, and biological factors that determine health according to priority. Using the Hanlon Method for prioritizing health problems, in consultation with local providers and the program director of La Brigada, five needs were identified: (1) improvements in access to specialized care from hand surgeons and (2) rehabilitation specialists; (3) improvements in upper extremity function; (4) access to transportation; and (5) improvement in physical activity and sports participation. Based on the results of this needs assessment, we learned that some of the needs were already part of the ongoing work of the partnership, but in addition, more needs became evident; for that reason, local health care providers and members of La Brigada identified potential solutions to these needs and are currently working to translate these in future interventions.

Complications and Cost of Syndactyly Reconstruction in the United States: Analysis of the Pediatric Health Information System.

BACKGROUND: Syndactyly is one of the most common congenital differences of the upper extremity and offers an exceptional opportunity to evaluate value-based care in pediatric orthopedic surgery. We designed a study to characterize complications and cost associated to syndactyly surgery among US pediatric hospitals. METHODS: A total of 2047 patients were identified for syndactyly surgery at 38 pediatric hospitals from 2009 to 2012 using the Pediatric Health Information System (PHIS) database. We examined costs as well as complication rates across hospitals stratified by patient and hospital variables. RESULTS: The postoperative complication rate was 1.9% (95% confidence interval [CI]: 1.3%-2.5%). Postoperative infection rate was 1.6% and surgical complication rate was 0.3%. Median adjusted standardized cost was $4112.5 (interquartile range: $2979-$6049). Patients with more than 1 diagnosis had 19 times higher risk of complications and were associated with 13% more hospital cost than those with syndactyly as single diagnosis ( P < .001). Finally, there was a wide variation in cost across hospitals; 8 (21%) yielded confidence limits above the benchmarked value. CONCLUSIONS: In the United States, it is important to recognize variations in practice of syndactyly surgery in hopes of developing quality improvement strategies in pediatric orthopedic surgery.